Background And Aims: X lymphoproliferative syndrome type 1 (XLP1) is a rare inborn error of immunity due to mutations of , encoding for slam-associated protein (SAP). The clinical phenotype includes severe mononucleosis, hemophagocytic lymphohistiocytosis (HLH), and B-cell lymphomas.
Methods: We report the case of a child affected with XLP1 who presented with an incomplete HLH, triggered by Epstein-Barr virus (EBV) and treated with rituximab, involving orbits and paranasal sinuses.
Ophthalmic Plast Reconstr Surg
July 2024
A primary orbital respiratory cyst is a congenital choristoma that presents in the orbit and with different signs and symptoms depending on the location, which might also change the surgical approach. The aim of this report is to describe 2 new cases of primary respiratory epithelial cysts and to review the literature on presentation, management, and risk factors with different surgical approaches and complications. Two cases presenting with gradually increased proptosis had a confirmed diagnosis of a respiratory epithelial cyst.
View Article and Find Full Text PDFObjectives: To evaluate the outcomes of strabismus surgery in patients with thyroid eye disease (TED) and to assess the influence of preceding treatments such as steroid administration, orbital decompression and radiotherapy.
Methods: Retrospective study on a cohort of patients with TED undergoing strabismus surgery. Data collection included clinical features, smoking status, preceding steroid therapy, orbital radiation therapy and orbital decompression.
Am J Ophthalmol Case Rep
March 2021
Purpose: Kaposi's sarcoma (KS) is a rare multi-centric vascular neoplasm, first described by Moritz Kaposi in 1872. It can appear in four different forms: classic, endemic, HIV-related and post-transplant form. We present an uncommon case of seronegative HIV infection patient with skin KS involving conjunctiva of both eyes.
View Article and Find Full Text PDFPurpose: Two new cases of primary cutaneous CD30+ anaplastic large-cell lymphoma (cALCL) of the eyelid are reported; these are analysed alongside existing cases to identify challenges relating to the diagnosis and management of such rare lesions.
Material And Methods: A review of existing literature on the PubMed database is conducted using the keywords: 'eyelid lymphoid proliferations', 'lymphoma of the eyelid', and 'primary cutaneous CD30+, ALK-anaplastic large-cell lymphoma of the eyelid'. Two new cases of cALCL are reported.
Background: The aim of this study was to analyze patients diagnosed, staged and treated for orbital and eyelid B-cell lymphoma (OEL).
Methods: One hundred and forty-one cases of OEL were included in this study. Primary endpoints were to analyze the histopathologic findings, the main risk factors and the type of treatment and to correlate them with recurrence of OEL.
Background: Solitary fibrous tumors (SFT), formerly called hemangiopericytoma, are rare tumors derived from mesenchymal cells originally described in the pleura, but these tumors may affect extraserosal tissues including the lacrimal gland and orbit.
Objective: Conduct a multi-centered clinical, radiological and histopathological analysis of 17 orbital SFT cases.
Design: A retrospective case series.
Purpose: To investigate the relation between the eighth edition of the American Joint Committee on Cancer staging system and histological risk classification for primary eyelid basal cell carcinoma.
Methods: Retrospective, observational case series of patients undergoing excisional biopsy for primary eyelid basal cell carcinoma in two tertiary centres between 2008 and 2018. Patients with <6 months of follow-up were excluded.
Purpose: To compare the binocular visual outcomes and satisfaction of patients with emmetropia or mild myopia (-0.50 diopters [D]) implanted with a bilateral spherical monofocal IOL.
Setting: Ophthalmology Department, Humanitas Clinical and Research Center, Rozzano, Milan, Italy.
Aims: The aim of this study is to assess the value of ultrasound in the differential diagnosis of orbital lesions.
Methods: One hundred and thirty-eight patients with clinical features of an orbital mass were examined by orbital ultrasound prior to undergoing surgery, from January 2000 to January 2017. All patients underwent excisional or incisional orbital biopsy.
Aim: Macular pigment density and microvascular density on optical coherence tomography angiography (OCTA) were measured in a cohort of type 1 diabetes mellitus (T1DM) patients with retinopathy in the attempt to shed light on the pathophysiology of this condition.
Methods: Eighty-two consecutive eyes of 59 patients with diabetic retinopathy examined at the Eye Clinic of the University of Naples Federico II from November 2016 to April 2017 were enrolled in this prospective study. Eighty normal eyes of 40 age-matched subjects without diabetes mellitus, without a history of glaucoma or evidence of intraocular surgery, and without retinal pathologic features constituted the control group.